Interleukin (IL-)1 is an important mediator of inflammatory responses and p
lays an important role in the pathogenesis of various autoimmune diseases.
Cicatricial pemphigoid (CP) is a multisystem autoimmune inflammatory diseas
e. We have studied the role of IL-1 in its pathogenesis. We have investigat
ed the serum levels of IL-1 components (IL-1 alpha, IL-1 beta, and IL-1Ra),
and determined the role of intravenous immunoglobulin (IVIg) therapy in pa
tients,vith CP, Serum levels of IL-1 alpha and beta were significantly high
er in untreated patients with active disease compared to levels in patients
in prolonged clinical remission and normal human controls (P<0.0001). The
serum levels of IL-1Ra were higher in patients in prolonged clinical remiss
ion compared to patients with active disease (P=0.002), Hence elevated leve
ls of IL-1<alpha> and beta and low levels of IL-1Ra correlate with disease
activity, The levels of IL-1 alpha and beta were statistically significantl
y higher in sera of CP patients with active disease pre-IVIg therapy compar
ed to post-IVIg therapy (P<0.0001). Statistically significantly higher leve
ls of IL-1Ra were present in post-IVIg treatment serum samples when compare
d to levels in pre-IVIg treatment (P<0.0001). In the in vitro experiments,
the levels of IL-1 alpha and beta produced by the peripheral blood mononucl
ear cells (PBMC) isolated from patients before IVIg therapy were significan
tly higher when compared to the PBMC isolated from post-IVIg patients (P<0.
0001). Significantly higher levels of IL-1Ra were observed in the supernata
nts of PBMC collected from pre-IVIg patients and cultured with exogenously
added IVIg, when compared to the levels of PBMC to which IVIg was not added
(P<0.0001). IL-1 may be an important cytokine involved in the pathogenesis
of CP, The regulation of IL-1 could be one of the mechanisms, amongst othe
rs, by which IVIg may exert its beneficial effect in the treatment of CP. (
C) Academic Press.